Correlation of thyroid and growth hormones to chromosomal instability in Egyptian Fanconi anemia patients.

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dc.contributor.authorAl-Haggar, Mohammaden_US
dc.contributor.authorAl-Morsy, Zakariaen_US
dc.contributor.authorYahia, Sohieren_US
dc.contributor.authorChalaby, Nehaden_US
dc.contributor.authorRagab, Amanyen_US
dc.contributor.authorMesbah, Abeeren_US
dc.date.accessioned2008-07-19en_US
dc.date.accessioned2009-05-30T12:34:39Z
dc.date.available2008-07-19en_US
dc.date.available2009-05-30T12:34:39Z
dc.date.issued2008-07-19en_US
dc.description.abstractOBJECTIVE: Fanconi anemia (FA) is a rare inherited genomic instability syndrome and usually associated with endocrine dysfunctions. We aimed to assess the diagnostic standards of chromosomal instability in FA and to correlate the breakage frequency with the severity of endocrinal dysfunctions. METHODS: Twenty seven FA patients were randomly selected from Hematology Unit of Mansoura University Children's Hospital; their mean age 8.8 yr. Sixteen normal children matched for age and sex were used as controls. Cytogenetic studies included peripheral blood lymphocyte cultures using phytohemagglutinin to obtain chromosomal spreads. Chromosomal breakage was induced by (i) Diepoxybutane 0.1 mug/ml. (ii) Mitomycin C 0.1 microg/ml. (iii) Irradiation of cultures to four radiation doses; 75, 150, 300 and 400 rads (rad1, rad2, rad3 and rad4 respectively). Chromosomal aberrations were scored from the previous 6 cultures besides a culture for spontaneous chromosomal breakage; then mean chromosomal breakage was calculated for the seven cultures. Endocrinal evaluation included quantitative determination of thyroid stimulating hormone (TSH) and tetraiodothyronine (T4), serum growth hormone (GH), insulin like growth factor-1 (IGF-1) and insulin levels. RESULTS: Chromosomal breakage was found to be significantly higher in patients than control when induced by Diepoxybutane (p = 0.003), Mitomycin (p = 0.001), rad3 (p = 0.043) and rad4 (p = 0.001). Mean chromosomal breakage was significantly negative correlated to head circumference (r = -0.57) and GH level (r = -0.50), with no significant correlation to other hormonal parameters. Mitomycin and rad4 were found more accurate than DEB test for diagnosis of FA in suspected cases. CONCLUSION: Correction of the frequently associated hormonal dysfunction (reduced GH and T4) should be considered in the treatment discipline of FA patients to improve their final height.en_US
dc.description.affiliationDepartment of Pediatrics, Mansoura University, Mansoura, Egypt. alhaggar@yahoo.co.uken_US
dc.identifier.citationAl-Haggar M, Al-Morsy Z, Yahia S, Chalaby N, Ragab A, Mesbah A. Correlation of thyroid and growth hormones to chromosomal instability in Egyptian Fanconi anemia patients. Indian Journal of Pediatrics. 2008 Jul; 75(7): 679-84en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/80825
dc.language.isoengen_US
dc.source.urihttps://medind.nic.in/icb/icbai.shtmlen_US
dc.subject.meshAdolescenten_US
dc.subject.meshCells, Cultureden_US
dc.subject.meshChilden_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshChromosomal Instability --geneticsen_US
dc.subject.meshChromosome Breakage --drug effectsen_US
dc.subject.meshDose-Response Relationship, Radiationen_US
dc.subject.meshEgypten_US
dc.subject.meshEpoxy Compounds --pharmacologyen_US
dc.subject.meshFanconi Anemia --geneticsen_US
dc.subject.meshFemaleen_US
dc.subject.meshGrowth Hormone --blooden_US
dc.subject.meshHumansen_US
dc.subject.meshInsulin-Like Growth Factor I --metabolismen_US
dc.subject.meshLymphocytesen_US
dc.subject.meshMaleen_US
dc.subject.meshMitomycin --pharmacologyen_US
dc.subject.meshMutagens --pharmacologyen_US
dc.subject.meshNucleic Acid Synthesis Inhibitors --pharmacologyen_US
dc.subject.meshThyroid Hormones --blooden_US
dc.subject.meshThyrotropin --blooden_US
dc.titleCorrelation of thyroid and growth hormones to chromosomal instability in Egyptian Fanconi anemia patients.en_US
dc.typeComparative Studyen_US
dc.typeJournal Articleen_US
dc.typeRandomized Controlled Trialen_US
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